The purpose of this study is to determine if Anakinra (an interleukin-1 receptor antagonist) can improve hearing thresholds in those patients with Autoimmune Inner Ear Disease (AIED) that did not respond to oral steroid therapy for a sudden decline in hearing. The patients to be enrolled will have recently completed a course of oral steroids and demonstrated no change in their audiometric thresholds following corticosteroid therapy.

The primary endpoint is to determine whether those treated with anakinra for 84 days demonstrate an improved hearing threshold compared with their pre-anakinra-treatment threshold. Audiometric thresholds will be compared to those treated with a prolonged corticosteroid taper and those that elect for no further treatment. The durability of the response will be measured over a total of 180 days.

Secondary Outcome Measures:

To assess the safety and tolerability of a three month (84 day) course of anakinra in corticosteroid resistant patients with Autoimmune Inner Ear Disease. [ Time Frame: 84 days ] [ Designated as safety issue: Yes ]

Estimated Enrollment:

38

Study Start Date:

June 2011

Estimated Study Completion Date:

June 2016

Estimated Primary Completion Date:

June 2016 (Final data collection date for primary outcome measure)

Intervention Details:

Drug: Anakinra

100mg of anakinra administered by a subcutaneous injection for 84 consecutive days.

Other Name: Kineret

Detailed Description:

Patients with immune mediated hearing loss (also known as autoimmune hearing loss) are typically treated with corticosteroids. Of those treated, approximately 60% respond, however, that response may be lost over time. Other therapies use to date have proven largely ineffectual in improving hearing. This study proposes a phase I open label clinical trial of Anakinra for corticosteroid-resistant patients to determine if this therapy is efficacious in hearing restoration.

Eligibility

Ages Eligible for Study:

13 Years to 75 Years

Genders Eligible for Study:

Both

Accepts Healthy Volunteers:

No

Criteria

Inclusion Criteria:

Bilateral sensorineural hearing loss with an active decline in hearing in one ear

No audiometric improvement with 28-30 days of oral prednisone or other corticosteroid, including an initial dose of 60mg per day for 14 days

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Please refer to this study by its ClinicalTrials.gov identifier: NCT01267994